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Cystic fibrosis

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 24 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.


● Autosomal recessive disorder affecting most critically the lungs; also pancreas, liver, intestines; characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions (Wikipedia)
Incidence: 1 in 20 in U.S. are carriers
● Most common mutation is #708 (seen in 70% with disease)
● Mutations cause reduced chloride ion in secretions, thicker respiratory secretions, upper respiratory infections
● Complications: pancreatic insufficiency late in disease course in 90%, diabetes, malabsorption, pancreatitis
● Mutations also cause defective cilia and infertility; may cause meconium ileus (5-10%), intussusception
● Cysts form secondary to ductal obstruction due to thick, tenacious secretions, variable numbers of cysts range from 1 to 3 mm, very rarely entire pancreas replaced by multiple macroscopic cysts, termed “pancreatic cystosis”

Micro images

At autopsy, pancreas is mucoid and slightly smaller than normal

Micro description

● Grade I: accumulation of secretion
● Grade II exocrine atrophy
● Grade III atrophy with lipomatosis
● Grade IV fibrosis with total obliteration of the exocrine glands and ducts with scattered islets of Langerhans

● Pancreatic ducts diffusely dilated and filled with numerous lamellated concretions
● Associated with fibrosis
● Nondiabetic patients have fibrocystic changes with normal islets, prominent nesidioblastosis, some persisting exocrine tissue
● Young adult diabetic patients have total loss of exocrine pancreas with fat replacement, no nesidioblastosis, reduced islets (Hum Pathol 1984;15:278)

Micro images

Abnormally viscid secretions from exocrine glands are round eosinophil masses distending and obstructing pancreatic ducts, leading to destruction of parenchyma and fibrosis

Positive stains

● PAS+ diastase resistant, CEA, alpha-1-antitrypsin (Arch Pathol Lab Med 1989;113:1142)


Differential diagnosis

● Kartegeners: defective cilia syndrome

End of Pancreas > Cysts > Cystic fibrosis

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